Understanding Pulmonary Arterial Hypertension

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A Serious Condition

PAH gets worse over time and can be serious, especially for people with scleroderma.

PAH was the leading cause of death, ahead of:
Other causes
in a registry of people with PAH associated with scleroderma from 2006 to 2016.

Who Is at Risk for PAH?

If you have scleroderma, you are at risk for PAH.

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5% to 19% of people with scleroderma will also develop PAH.

Black people with scleroderma may develop PAH more frequently than the overall population of people with scleroderma.

The rate of PAH associated with scleroderma was higher in Black people (48%) vs the overall population (39%).*

*Results based on a 2003 study of 709 people with scleroderma that included 130 Black people.

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Risk Factors for PAH

Additional factors that can increase your risk include:



Older age at the time of your scleroderma diagnosis



If you have had scleroderma for a long time

Other conditions

Raynaud’s phenomenon
Raynaud’s phenomenon

Small blood vessels in the hands and toes spasm in the cold

Cutaneous telangiectasias
Spider veins

Spider veins (also known as telangiectasias) may appear on skin or anywhere in the body

Digital ischemia
Digital ischemia

Painful pale, white, or blue fingers

Limited cutaneous sclerosis
Limited cutaneous scleroderma

A type of scleroderma in which skin hardening and tightening happens only on the fingers, hands, forearms, and face

Test results

Your doctor can tell you if your test results indicate risk.

Blood tests show certain antibodies

Blood tests show certain antibodies

Results in the low range on certain pulmonary function tests

Results in the low range on certain lung function tests

What Is PAH?

PAH is a disease of high blood pressure in the lungs that can be more common in people with scleroderma.

Find out more

Talk to Your Rheumatologist

Ask your rheumatologist about yearly screening.

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